Conductive and Cochlear Hearing Loss

  • Tobias KleinjungEmail author


  1. 1.

    Any kind of hearing loss may be accompanied by tinnitus.

  2. 2.

    This chapter describes possible causes of conductive hearing loss located in the external ear and middle ear.

  3. 3.

    Pathologies of this area include neoplastic changes (e.g., tumors), inflammatory disease (e.g., otitis media), or disorders of unknown origin (e.g., otosclerosis).

  4. 4.

    Cochlear hearing loss of genetic origin can be classified in to syndromic and non-syndromic forms.

  5. 5.

    Labyrinthitis can occur due to bacterial or viral infection or in the context of immunological disease.



Hearing loss Inner ear Middle ear Otitis media Otosclerosis Tinnitus 



Autoimmune inner ear disease


Non-syndromic hearing loss


Syndromic hearing loss


  1. 1.
    Takemori S, Tanaka Y, Suzuki JI. Thalidomide anomalies of the ear. Arch Otolaryngol, 1976;102(7):425–7PubMedCrossRefGoogle Scholar
  2. 2.
    Ostrowski VB, Wiet RJ. Pathologic conditions of the external ear and auditory canal. Postgrad Med, 1996;100(3):223–8, 233–7PubMedCrossRefGoogle Scholar
  3. 3.
    Adams WS. The aetiology of swimmer’s exostoses of the external auditory canals and of associated changes in hearing. J Laryngol Otol, 1951;65(4):232–50; concl.PubMedCrossRefGoogle Scholar
  4. 4.
    Mlynski R, Radeloff A, Brunner K, Hagen R. [Exostoses of the external auditory canal Is the cold water hypothesis valid for patients in continental areas?] HNO, 2008;56(4):410–6. GermanPubMedCrossRefGoogle Scholar
  5. 5.
    Van Gilse, PHG. Des observations ultérieures sur la genèse des exostoses du conduit externe par l’irritation d’eau froide. Acta Otolaryngol, 1938;2:343–352CrossRefGoogle Scholar
  6. 6.
    Breau RL, Gardner EK, Dornhoffer JL. Cancer of the external auditory canal and temporal bone. Curr Oncol Rep, 2002;4(1):76–80PubMedCrossRefGoogle Scholar
  7. 7.
    Horbelt CV. A review of physical, behavioral, and oral characteristics associated with Treacher Collins syndrome, Goldenhar syndrome, and Angelman syndrome. Gen Dent, 2008;56(5):416–9PubMedGoogle Scholar
  8. 8.
    Rice DP. Clinical features of syndromic craniosynostosis. Front Oral Biol, 2008;12:91–106PubMedCrossRefGoogle Scholar
  9. 9.
    Jahrsdoerfer RA, Yeakley JW, Aguilar EA, Cole RR, Gray LC. Grading system for the selection of patients with congenital aural atresia. Am J Otol, 1992;13(1):6–12PubMedGoogle Scholar
  10. 10.
    Timmermans K, Vander Poorten V, Desloovere C, Debruyne F. The middle ear of cleft palate patients in their early teens: a literature study and preliminary file study. B-ENT, 2006;2(Suppl 4):95–101PubMedGoogle Scholar
  11. 11.
    Phua YS, Salkeld LJ, de Chalain TM. Middle ear disease in children with cleft palate: protocols for management. Int J Pediatr Otorhinolaryngol, 2009;73(2):307–13PubMedCrossRefGoogle Scholar
  12. 12.
    Chole RA, McKenna M. Pathophysiology of otosclerosis. Otol Neurotol, 2001;22(2):249–57PubMedCrossRefGoogle Scholar
  13. 13.
    Altmann F, Glasgold A, Macduff JP. The incidence of otosclerosis as related to race and sex. Ann Otol Rhinol Laryngol, 1967;76(2):377–92PubMedGoogle Scholar
  14. 14.
    Markou K, Goudakos J. An overview of the etiology of otosclerosis. Eur Arch Otorhinolaryngol, 2009;266(1):25–35PubMedCrossRefGoogle Scholar
  15. 15.
    Ali IBH, Thys M, Beltaief N, Schrauwen I, Hilgert N, Vanderstraeten K, Dieltjens N, Mnif E, Hachicha S, Besbes G, Arab SB, Van Camp G. A new locus for otosclerosis, OTSC8, maps to the pericentromeric region of chromosome 9. Hum Genet, 2008;123(3):267–72CrossRefGoogle Scholar
  16. 16.
    Niedermeyer HP, et al., Measles virus and otosclerosis. Adv Otorhinolaryngol, 2007;65:86–92PubMedGoogle Scholar
  17. 17.
    Lippy WH, Berenholz LP, Schuring AG, Burkey JM. Does pregnancy affect otosclerosis? Laryngoscope, 2005;115(10):1833–6PubMedCrossRefGoogle Scholar
  18. 18.
    Dahlqvist A, Diamant H, Dahlqvist SR, Cedergren B. HLA antigens in patients with otosclerosis. Acta Otolaryngol, 1985;100(1–2):33–5PubMedCrossRefGoogle Scholar
  19. 19.
    Gristwood RE, Venables WN. Otosclerosis and chronic tinnitus. Ann Otol Rhinol Laryngol, 2003;112(5):398–403PubMedGoogle Scholar
  20. 20.
    Sobrinho PG, Oliveira CA, Venosa AR. Long-term follow-up of tinnitus in patients with otosclerosis after stapes surgery. Int Tinnitus J, 2004;10(2):197–201PubMedGoogle Scholar
  21. 21.
    Sellari-Franceschini S, Ravecca F, De Vito A, Berrettini S. [Progressive sensorineural hearing loss in cochlear otosclerosis]. Acta Otorhinolaryngol Ital, 1998;18(4 Suppl 59):59–65PubMedGoogle Scholar
  22. 22.
    Shinkawa A, Sakai M, Ishida K. Cochlear otosclerosis 30 years after stapedectomy confirmed by CT, MRI. Auris Nasus Larynx, 1998;25(1):95–9PubMedCrossRefGoogle Scholar
  23. 23.
    Linthicum F, Jr. Post-stapedectomy cochlear otosclerosis. Ear Nose Throat J, 2009;88(4):872PubMedGoogle Scholar
  24. 24.
    Hinojosa R, Marion M. Otosclerosis and sensorineural hearing loss: a histopathologic study. Am J Otolaryngol, 1987;8(5):296–307PubMedCrossRefGoogle Scholar
  25. 25.
    Ramsden R, Rotteveel L, Proops D, Saeed S, van Olphen A, Mylanus E. Cochlear implantation in otosclerotic deafness. Adv Otorhinolaryngol, 2007;65:328–34PubMedGoogle Scholar
  26. 26.
    Mafee MF. Use of CT in the evaluation of cochlear otosclerosis use of CT in the evaluation of cochlear otosclerosis. Radiology, 1985;156(3):703–8PubMedGoogle Scholar
  27. 27.
    Alford BR, Guilford FR. A comprehensive study of tumors of the glomus jugulare. Laryngoscope, 1962;72:765–805PubMedCrossRefGoogle Scholar
  28. 28.
    Gulya AJ. The glomus tumor and its biology. Laryngoscope, 1993;103(11 Pt 2 Suppl 60):7–15PubMedGoogle Scholar
  29. 29.
    Horn KL, Hankinson H. (1994) Tumors of the jugular foramen, in Neurotology, RK Jackler, DE Brackmann, Editors, Mosby: New York, 1059–64Google Scholar
  30. 30.
    Sismanis A. (1997) Pulsatile tinnitus, in Clinical otology, GB Hughes, ML Pensak, Editors, Thieme: New York, 445–60Google Scholar
  31. 31.
    Wackym PA, Friedman I. (2000) Unusal tumors of the middle ear and mastoid, in Tumors of the ear and temporal bone, RK Jackler, CLW Driscoll, Editors, Lippincott Williams & Wilkins: Philadelphia, 128–45Google Scholar
  32. 32.
    Birkenhager R, Aschendorff A, Schipper J, Laszig R. [Non-syndromic hereditary hearing impairment]. Laryngorhi nootologie, 2007;86(4):299–309; quiz 310–3PubMedCrossRefGoogle Scholar
  33. 33.
    Kitamura K, Takahashi K, Tamagawa Y, Noguchi Y, Kuroishikawa Y, Ishikawa K, Hagiwara H. Deafness genes. J Med Dent Sci, 2000;47(1):1–11PubMedGoogle Scholar
  34. 34.
    Bayazit YA, Yilmaz M. An overview of hereditary hearing loss. ORL J Otorhinolaryngol Relat Spec, 2006;68(2):57–63PubMedCrossRefGoogle Scholar
  35. 35.
    Smith RJ, Bale JF, Jr, White KR. Sensorineural hearing loss in children. Lancet, 2005;365(9462):879–90PubMedCrossRefGoogle Scholar
  36. 36.
    Hess C, Rosanowski F, Eysholdt U, Schuster M. [Hearing impairment in children and adolescents with Down’s syndrome]. HNO, 2006;54(3):227–32PubMedCrossRefGoogle Scholar
  37. 37.
    Batissoco AC, Abreu-Silva RS, Braga MC, Lezirovitz K, Della-Rosa V, Alfredo T, Jr, Otto PA, Mingroni-Netto RC. Prevalence of GJB2 (connexin-26) and GJB6 (connexin-30) mutations in a cohort of 300 Brazilian hearing-impaired individuals: implications for diagnosis and genetic counseling. Ear Hear, 2009;30(1):1–7PubMedCrossRefGoogle Scholar
  38. 38.
    Douglas SA, Sanli H, Gibson WP. Meningitis resulting in hearing loss and labyrinthitis ossificans – does the causative organism matter? Cochlear Implants Int, 2008;9(2):90–6PubMedCrossRefGoogle Scholar
  39. 39.
    Hviid A, Rubin S, Muhlemann K. Mumps. Lancet, 2008;371(9616):932–44PubMedCrossRefGoogle Scholar
  40. 40.
    Sweeney CJ, Gilden DH. Ramsay Hunt syndrome. J Neurol Neurosurg Psychiatry, 2001;71(2):149–54PubMedCrossRefGoogle Scholar
  41. 41.
    Sugiura M, Naganawa S, Nakata S, Kojima S, Nakashima T. 3D-FLAIR MRI findings in a patient with Ramsay Hunt syndrome. Acta Otolaryngol, 2007;127(5):547–9PubMedCrossRefGoogle Scholar
  42. 42.
    McCabe BF. Autoimmune sensorineural hearing loss. Ann Otol Rhinol Laryngol, 1979;88(5 Pt 1):585–9PubMedGoogle Scholar
  43. 43.
    Ruckenstein MJ. Autoimmune inner ear disease. Curr Opin Otolaryngol Head Neck Surg, 2004;12(5):426–30PubMedCrossRefGoogle Scholar
  44. 44.
    Broughton SS, Meyerhoff WE, Cohen SB. Immune-mediated inner ear disease: 10-year experience. Semin Arthritis Rheum, 2004;34(2):544–8PubMedCrossRefGoogle Scholar
  45. 45.
    Bovo R, Ciorba A, Martini A. The diagnosis of autoimmune inner ear disease: evidence and critical pitfalls. Eur Arch Otorhinolaryngol, 2009;266(1):37–40PubMedCrossRefGoogle Scholar
  46. 46.
    Cruickshanks KJ, Wiley TL, Tweed TS, Klein BE, Klein R, Mares-Perlman JA, Nondahl DM. Prevalence of hearing loss in older adults in Beaver Dam, Wisconsin The Epidemiology of Hearing Loss Study. Am J Epidemiol, 1998;148(9):879–86PubMedCrossRefGoogle Scholar
  47. 47.
    Mazurek B, Stöver T, Haupt H, Gross J, Szczepek A. [Pathogenesis and treatment of presbyacusis current status and future perspectives]. HNO, 2008;56(4):429–32; 434–5PubMedCrossRefGoogle Scholar
  48. 48.
    Rosenhall U, Karlsson AK. Tinnitus in old age. Scand Audiol, 1991;20(3):165–71PubMedCrossRefGoogle Scholar
  49. 49.
    Nondahl DM, Cruickshanks KJ, Wiley TL, Klein R, Klein BE, Tweed TS. Prevalence and 5-year incidence of tinnitus among older adults: the epidemiology of hearing loss study. J Am Acad Audiol, 2002;13(6):323–31PubMedGoogle Scholar
  50. 50.
    do Carmo LC, Médicis da Silveira JA, Marone SA, D’Ottaviano FG, Zagati LL, Dias von Söhsten Lins EM. Audiological study of an elderly Brazilian population. Braz J Otorhinolaryngol, 2008;74(3):342–9Google Scholar
  51. 51.
    Ahmad N, Seidman M. Tinnitus in the older adult: epidemiology, pathophysiology and treatment options. Drugs Aging, 2004;21(5):297–305PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Department of OtorhinolaryngologyUniversity of RegensburgRegensburgGermany

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